IgG4-Related Lung Disease Presenting as a Consolidative Mass: A Case Report

Ha Young Park; Joungho Han; Guhyun Kang; Chin A Yi; Man Pyo Chung

doi:10.6058/jlc.2010.9.2.103

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Park, Han, Kang, Yi, and Chung: IgG4-Related Lung Disease Presenting as a Consolidative Mass: A Case Report

Original Article

J Lung Cancer 2010;9(2):103-105.

Published online: 10 January 2010

DOI: https://doi.org/10.6058/jlc.2010.9.2.103

IgG4-Related Lung Disease Presenting as a Consolidative Mass: A Case Report

Ha Young Park¹, Joungho Han¹, Guhyun Kang¹, Chin A Yi², Man Pyo Chung³

¹Departments of Pathology

²Radiology and Center for Imaging Science

³Division of Pulmonary and Critical Care Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea

Address for correspondence Joungho Han, M.D. Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50, Irwon-dong, Gangnam-gu, Seoul 135-710, Korea Tel: 82-2-3410-2800 Fax: 82-2-3410-0025 E-mail: hanjho@skku.edu

Received 8 September 2010 Revised 15 September 2010 Accepted 16 September 2010

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Immunoglobulin G4 (IgG4)-related sclerosing disease involving the lung is a rare condition, and this is characterized by an elevated serum IgG4 level, fibrotic inflammation with numerous IgG4-positive plasma cells and a response to steroid therapy. We present here a case of pulmonary IgG4-related disease in a 75-year-old man who presented with cough and yellowish sputum for the previous 3 months. The chest images showed a consolidative mass in the right lower lobe that suggested mucinous bronchioloalveolar carcinoma. The wedge resected specimen revealed an ill-defined graytan, firm lesion. Microscopically, the lesion showed a diffuse lymphoplasmacytic infiltration with irregular fibrosis in the alveolar interstitium and bronchovascular bundles. There were numerous IgG4-positve plasma cells and these cells were diffusely distributed. The serum IgG4 level was elevated on the postoperative check-up (249 mg/dL). After corticosteroid therapy for 7 months, the patient's symptoms and radiologic abnormalities were improved.

Keywords: Autoimmune disease, IgG4, Interstitial lung disease, Plasma cells, Lung neoplasms

References

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Figures

Fig. 1.

(A) The chest X-ray shows parenchymal consolidation at the right costophrenic angle with pleural effusion. (B, C) The chest computed tomography scans reveal air space consolidation and ground-glass attenuations.

Fig. 2.

(A, B) Scanning views of the VATS wedge resected specimen show a subpleural patchy mononuclear cells infiltration. (C) The low power view shows a dense lymphoid cell infiltration with fibrosis in the alveolar interstitium and bronchovascular bundles (H&E satin, ×40). (D) The lesion is composed of mature plasma cells and some lymphocytes (H&E stain, ×200). (E) Phlebitis is also present in the interlobular septal vein (H&E stain, ×100). (F) Many plasma cells are positive for IgG4 (×400).

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